Lesson 10

Collagenoses. Lupus erythematosus. Sclerodermia. Etiology, pathogenesis. Clinical features. Diagnostics. Treatment. Prevention.

1. Theme urgency

Isolation of the group of collagenoses is merely of relative significance today. The cardinal feature of these conditions is inflammation in the connective tissue which leads to dermal atrophy or sclerosis, to arthritis, and sometimes to abnormalities in other organs. In addition, antibodies form against normal tissues and cellular components; these disorders are therefore classed as autoimmune. The main connective tissue disorders present as a spectrum ranging from the benign cutaneous variants to severe multisystem diseases.

Lupus erythematosus (LE) is a good example of such a spectrum, ranging from the purely cutaneous type (discoid LE), through patterns associated with some internal problems (disseminated discoid LE and subacute cutaneous LE), to a severe multisystem disease (systemic lupus erythematosus, SLE).

Scleroderma is a chronic disease of unknown etiology that affects the microvasculature and loose connective tissue and is characterised by fibrosis and obliteration of vessels in the skin, lungs, gastrointestinal tract, kidneys and heart. It may occur in a localised form or as a systemic disease.

Dermatomyositis is a subset of polymyositis with distinctive skin changes. The cause is unknown but an autoimmune mechanism seems likely. Autoantibodies to striated muscle are found. When starting after the age of 40, dermatomyositis may signal an internal malignancy. Presumably, the epitopes of some tumour antigens are so similar to those of muscle antigens that antibodies directed against the tumour cross-react with muscle cells and initiate the disease in a few adults with internal malignancy.

2. Concrete Objectives:

Students must know:

1. Etiology and pathogenesis of connective tissue disorders (lupus erythematosus and scleroderma).

2. Classification of connective tissue disorders (lupus erythematosus and scleroderma).

3. Clinical features of connective tissue disorders (lupus erythematosus and scleroderma).

4. Morphology of connective tissue disorders (lupus erythematosus and scleroderma).

5. Investigations of connective tissue disorders (lupus erythematosus and scleroderma).

6. Diagnosis of connective tissue disorders (lupus erythematosus and scleroderma).

7. Treatment of connective tissue disorders (lupus erythematosus and scleroderma).

Students should be able to:

1. To collect the medical history of patient with diseases of the connective tissue.

2. To diagnostic diseases of the connective tissue in patient in typical case.

3. To determine the main signs of discoid lupus erythematosus (“ladies’ heel” sign and Besnier-Meshchersky’s sign).

4. To prescribe the treatment for patient with diseases of the connective tissue in typical case.

5. To recommend preventive measures for patient with diseases of the connective tissue.

3. Tasks for self-study during preparation for lesson.